Human Population of the Balearic Island: the Case of Chuetas and Ibizans*

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CONTRIBUTIONS to SCIENCE, 4 (1): 85-91 (2008) Institut d’Estudis Catalans, Barcelona DOI: 10.2436/20.7010.01.39 ISSN: 1575-6343 distinguished lectures www.cat-science.cat

Human population of the Balearic Island: the case of Chuetas and Ibizans*

Misericòrdia Ramon**, Antònia Picornell, José A. Castro Laboratori de Genètica, Departament de Biologia, Universitat de les Illes Balears

Misericòrdia Ramon graduated from Biology from the Autonomous University of Barcelona (UAB) in 1975 and obtained her doctorate from the University of the Balearic Islands in 1981, where she is an Associate Professor in Genetics since 1985. Her teaching is concentrated in Genetics, Human Genet- ics and Evolution, which she teaches both at a bachelor’s level and in the Biology and Medicine doctor- ates. She has also taught in several Master degrees, and in specialist and postgraduate courses, during the past 20 years. As a researcher, she has worked in population genetics, and her research is concentrated in the study of human populations of the Balearic Islands. She has also studied the evolution mechanisms of other species, such as lizards, taking place in the Islands. She is a coauthor of more than 60 publications and of more than 70 communications in congresses. She has organized activities for divulging science and sustains collaborations with various recognized Spanish and international research centers.

Introduction The Balearic Islands offer an opportunity to observe genetic diversity based on reproductive isolation: geographical isola- The Balearic Archipelago is located in the western Mediterra- tion (Ibiza population), and cultural isolation (Chuetas, descend- nean Sea, near the eastern coast of the Iberian Peninsula. It is ents of Majorcan Jews). The three main islands have different composed of three major islands, Majorca, Minorca, and Ibiza. histories of settlement. Majorca and Minorca have similar meg- Due to their geographical position in the Mediterranean Sea, alithic monuments and were occupied by the same people in the Balearic Islands have been settled throughout their history historical periods. Ibiza is different from the two other islands, by people of different populations. Merchant Greek and Cartha- not only in its landscape and vegetation but also in the origin of ginian ships sailed to trade with the aboriginal Balearics. Vari- the founding settlements. Ibiza was an important Carthaginian ous civilizations have left their mark on the islands, including colony, evidence of which can be seen in archaeological re- the Phoenicians, Greeks, Carthaginians, Romans, Vandals, By- mains that have been found. The Ibizan population was repro- zantines, and Moors. In 654 BC, the Phoenicians made Ibiza ductively isolated and has thus received little gene flow from (Ibusim) an important commercial centre. In 123 BC, Romans outside. Although the scarcity of available data concerning conquered Majorca and made it part of Roman history for five population size (e.g., 3000 in 1392; 9596 in 1652) does not in- and a half centuries. The Western Byzantines became the new dicate a drastically low number of people, the incidence of in- conquerors in 534 AD, until they were ousted by the Muslims in fectious diseases, such as the plague and malaria, considera- the 8th century. In 902 AD, the entire archipelago was annexed bly reduced the effective size of the population. Moreover, the to the Caliphate of Cordoba. At the end of 1229, Jaume I ‘the demographic structure and marriage practices highlight the ex- Conqueror’, the Catalan King of the Crown of Aragon, annexed istence of genetic drift on this island. The rural population had a Majorca to his kingdom. The other islands were also conquered dispersed township with small properties, and marriages were and occupied by the Catalans in the following years, thus form- suitably arranged so as not to divide the lands, and in numer- ing the basis of the present day islands. At the end of the 15th ous cases they were carried out between relatives. This appar- century, the Balearics were united with the Kingdom of Spain ent reproductive isolation was disturbed by the 1970s tourist as part of the political union of Castilla and Aragon. influx that considerably increased the total population. The genetic structure of the present-day Jewish population is the outcome of a common ancestral gene pool and the admixture with people among whom the Jews lived. A number of * Based on the Lecture given by Misericòrdia Ramon at the Institute Jews living in the Middle East moved west in 70 AD after the for Catalan Studies, Barcelona, on 6 April 2006. Romans destroyed Jerusalem. The first Jewish communities ** Author for correspondence: Misericòrdia Ramon, Institut Univer- were established in sitari d’Investigacions en Ciències de la Salut i Laboratori de Genètica, Departament de Biologia, Universitat de les Illes Balears. 07122 Palma Majorca in the 1st century AC. They reached a period of de Mallorca, EU. Tel. +34 971173152. Email: [email protected] great development and scholarly achievement throughout

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Spain and Portugal. The Sephardic Jews, who were descend- other Balearic populations [19]. For some markers the Ibiza ants of Jews whose ancestors lived in the Iberian Peninsula, population seems to be closer to North African, Middle East- were thrown out of Spain in 1492 (Sephardi meaning ‘from ern, and other insular Mediterranean populations than to Euro- Sepharad’, which is Hebrew for ‘Spain’). Those who stayed in pean populations. It is thought that this might be due to the Spain were forced to convert to Christianity between 1391 and different origin of the Ibiza population. 1497. After the expulsion edict they left and settled mainly in Mitochondrial DNA (mtDNA) is a double-stranded, circular, North Africa, the Balkans and Eastern Mediterranean countries. covalently-closed molecule of 16569 bp in humans that is inher- The Spanish Inquisition began its activities in ited as a haploid from the mother without recombination and/or Majorca in 1488 and as a result, 769 converted individuals segregation. Its high mutation rate (2-4% per million years) leads were judged during the following 50 years. In 1678 and 1691 to a high degree of variability between individuals. Thus mtDNA the Spanish Inquisition acted again in Majorca conducting an is a good tool for studying the genetic structure of populations ‘auto de fe’ (act of faith), which condemned 240 individuals be- [3]. The first human population studies were performed by mtD- longing to this community. The descendants of this Majorcan NA restriction enzyme analyses, and they revealed differences Jewish population, the Chuetas, were secluded by their imme- between the four ethnic groups (Caucasian, Amerindian, African, diate neighbours and have shown a strong endogamic behav- and Asian). In the Balearic Islands, the study of the genetic struc- ior from this period until nowadays. This isolation almost exclu- ture of these populations through restriction fragment length sively affected the individuals with the 15 typical surnames polymorphism (RFLP) analysis of their mtDNA by using 5 restric- belonging to the accused people in the last Inquisition. tion enzymes showed similar results to previous studies of the genetic structure of the Balearic population using hematologic polymorphisms [14,19]. The genetic homogeneity is higher in Genetic variation in the population of Ibiza (Spain) Majorca than in Minorca and Ibiza. This result is due to the high vs. other Balearic Islands by several genetic frequency of haplotype 1 in this population. In Minorca, with the population markers most heterogeneous population, the most frequent haplotype is also 1, although there are other haplotypes in polymorphic fre- Allele frequencies were calculated for the following blood group quencies as well as a new one, 150 (8%). Ibiza had only four and serum systems: ABO, Rh, MNSs, P, Lewis, Duffy, Kell, haplotypes, with haplotype 1 also having the highest frequency. ORM, GC, TF, PI, and HP. The allele frequencies from Ibiza The genetic diversity between populations is about seven fold were compared with those from other Balearic Islands (Majorca higher for Majorca–Ibiza and Minorca–Ibiza than for Majorca– and Minorca) and with related European and North African Minorca, which indicates a genetic differentiation of Ibiza with groups. Data revealed that Ibiza is genetically different from the respect to Majorca and Minorca (Table 1 / Table 1 IM 1998).

Table 1. Frequencies (in percentage) and haplotypic diversities of the different haplotypes detected in the Balearic Islands [3]

Majorca Minorca Ibiza Chuetas

Types of mtDNA Haplotype N % N % N % N %

1 2-1-1-1-1 44 84.6 28 56.0 25 50.0 39 72.2 2 3-1-1-1-3 0 − 0 − 5 10.0 0 − 6 2-1-2-1-1 2 3.8 7 14.0 0 − 7 13.0 7 3-1-1-1-1 1 1.9 0 − 0 − 0 − 8 1-1-1-1-1 0 − 3 6.0 0 − 0 − 11 2-2-3-1-5 0 − 0 − 1 2.0 0 − 18 2-3-1-4-9 3 5.8 0 − 19 38.0 4 7.4 22 2-3-1-1-9 1 1.9 0 − 0 − 0 − 24 2-1-1-4-2 0 − 1 2.0 0 − 0 − 39 2-1-4-1-1 0 − 0 − 0 − 1 1.8 56 2-1-1-1-6 0 − 1 2.0 0 − 0 − 57 2-3-1-4-13 1 1.9 6 12.0 0 − 2 3.7 59 2-1-1-1-20 0 − 0 − 0 − 1 1.8 150 2-1-2-1-6 0 − 4 8.0 0 − 0 − Total 52 100 50 100 50 100 54 100 h 0.284 0.655 0.607 0.444

h, haplotypic diversity. N, number of individuals.

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Considering the different haplotypes, the most characteristic in Ibiz a Majorca are Caucasians (mainly haplotype 1 and also haplotypes 6 and 18), although the presence of haplotype 7 suggests an Spain Arabian/African genetic contribution to this population. Haplo- M inorca type 7 could have been introduced into Majorca by the Arabians, C atalonia 50 who lived in the islands for around five hundred years. This hap- M ajorca lotype is not as frequent in Majorca (1.9%) as it is in Arabian com- F rance munities (4.8%) [21] but it is in the same order as in other Cauca- 55 Po rtugal sian groups such as the Romans (1.1%) [2] and Askenazi Jews Ashkenazim Jews

(1.3%) [21]. In Ibiza, the exceptional fact is the high frequency of Leba nese Jews

haplotype 18 (38%). This is considered an ancient Mediterrane- Chuetas an mtDNA type found at a maximum frequency of 12% in the Iraki Jews

centre and south of the Italian peninsula and Sicily. We can say 81 Moroccan Jews that, in Ibiza, two mtDNA lineages are present, one represented Algeria

by haplotype 1 and the other by haplotype 18 [3]. Sardinia The HLA system was another marker extensively studied 56 Italy from an evolutionary perspective. The region contains a number of closely linked genes whose products control a variety of func- Figure 1. Tree base on Standard Genetic Distances using the frequen- tions concerned with the regulation of immune responses. cies of HLA-A, -B and -DRBI. Only the bootstrap above 50 are represented. Genes comprising the major histocompatibility complex (MHC) play a central role in governing the immune response of verte- brates. A great deal of information has been revealed on the molecular biology and physiology of these loci, but three fea- tures—the high polymorphism, tight linkage among the loci, and the nonrandom association of alleles—make the system of particular interest from the perspective of population genetics. In- formation on the dynamic evolutionary forces that have acted on a locus can be inferred from the number and distribution of alleles that it carries. Five loci from the HLA region of the human MHC (HLA-A, -B, -Cw, -DRB1 and -DQB1 loci) have been ex- amined [5]. Some peculiarities were observed in the distribution of common haplotypes among the three main Balearic Islands. The Ibizan population was genetically different from the other Balearic populations, with a high frequency of some haplotypes, Figure 2. Haptoglobin subtypes. Genotypes in lines 1,2,12:1S-2FS; for example, A29-Cw*16-B44-DRB1*07-DQB1*03; A1-Cw*07- lines 3,8,9: 2FS; lines 4,6,11:2FS-1F; line 5:2SS-1F; line 7:1F; 10:2SS- FS. B8-DRB1*03-DQB1*02. We also found a new haplotype, A25- Cw*12-B39-DRB1*11-DQB1*03 (3.5%), in Ibizans and a more limited variability in the HLA alleles that were expressed, per- relatively low frequency in Majorcans (2%) and Minorcans haps because of genetic isolation. The genetic diversity of the (1.5%). Interestingly, it had a very high frequency in the neigh- populations from Majorca and Minorca were similar and more boring Mediterranean regions such as Algeria, where it was the related to the mainland Spanish population. Interestingly, our re- most frequent haplotype [4], Sardinia (12.5%) [8], and in the sults show a frequent haplotype, A25-Cw*12-B39-DRB1*11- Basque region (3.7%) (Figure 1 / Figure 2; [5]). DQB1*03, found in Ibiza (3.5%), that is not found in other populations and might be specific to the original population of the island (Founder effect) (Figure 1 / Figure 2; [5]). We can specu- Genetic variability of Chuetas (Majorcans Jews): a late about its Phoenician/Carthaginian origin although the avail- comparative study able data does not support this as a similar haplotype was not found in populations belonging to countries that occupy the A study of the Chueta population has been carried out to es- geographic territory from where the Phoenicians came [5]. The tablish the genetic relationships between the Chuetas and oth- alleles A25 and B39 are relatively frequent in neighboring popu- er Jewish and non-Jewish circum Mediterranean populations lations such as Italians (3% and 2%), Portuguese (1.9% and by several population-variability markers. 2.9%), Spaniards (1.5% and 1%) and French (1.4% and 2.5%), Picornell, et al. (1997) studied the Chueta community (iden- and rare in other Mediterranean Island populations such as Cor- tified by on of the 15 surnames: Aguiló, Bonnin, Cortés, Forte- sica (1% and 0.5%) and Sardinia (0% and 1%). A30-Cw*05- za, Fuster, Martí, Miró, Picó, Piña, Pomar, Segura, Tarongí, B18, frequently found in Spaniards and defined as of Iberian Valentí, Valleriola and Valls) using blood groups ABO, MNSs, P, Paleo-North African origin [1,15], was not found in Ibizans (the Ph, Kell (KEL), and Duffy (FY); red cell enzymes glucose 6-phos- most southern island of Balearic Islands) and was found at a phate dehydrogenase (G6PD), 6-phosphogluconate dehydro-

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genase (PGD), acid phosphatase (ACP1), phosphogluco- Differential maternal and paternal contributions to mutase 1 (PGM1), glyoxalase 1 (GLO1), and adelynate kinase the genetic pool of Balearic Archipelago (AK1); serum proteins haptoglobin (HP) and group specific component (CG). The results indicate that most of the Jewish It was observed in most studies that there is a different contri- communities resemble each other and other Middle Eastern bution of Y-chromosome and mtDNA haplotypes to the signifi- populations, because of their common origin, but they also cant differences between populations, which can be explained have affinities with their host peoples. The Chueta population is by unequal involvement of males and females in the different midway between non-Jewish populations of the western Medi- admixtures. The expansion and loss of matrilines and patrilines terranean and some Jewish populations, especially European can cause considerable fluctuation in the frequencies of mtD- ones (Polish, Askhenazi, and Sephardic Jews), although they NA and Y-chromosome haplotypes (Helgason, et al., 2003). In are closer to non-Jewish populations [16]. (Figure 2 / Figure 3, general, lower levels of Y-chromosome diversity than mtDNA Picornell article, 1997). As expected, the Samartians are clearly diversity are reported for the same population, and this reflects separated, and it is interesting to see the proximity of the Ethio- patrilocality and/or biased male reproductive success [11], al- pian populations and Ethiopian Jews. In fact, the Ethiopian though in some populations the inverse process is produced Jews were converted and, so they have the same genetic and matrilocality has also been considered [22]. structure as the rest of the Ethiopians. We have compared the genetic diversity between Ibiza and Most mtDNA variation studies have employed one of two the population of the other Balearic Islands and also between methods: direct sequencing of the rapidly evolving control re- the Archipelago with respect to circum-Mediterranean popula- gion (CR), or digestion of the entire molecule by means of tions. For such a comparison we studied autosomal and Y- standard sets of restriction enzymes. Richards et al. (1996) chromosome STRs, as well as mtDNA sequence data analyzed produced a phylogenetic network of sequences from the first from the same individuals. The analysis of 9 autosomal STRs hypervariable segment (HVRI) of the CR. The analysis of the (D3S1358, vWA, FGA, D8S1179, D21S11, D18S51, D5S818, mtDNA hypervariable region segment I sequence (HVRI) in D13S317, and D7S820) showed that Ibiza had significant dif- three insular Balearic populations (Majorca, Minorca, and Ibiza) ferentiation with respect to other Balearic populations and also and the Chueta community showed that the incidence of with respect to insular and continental populations from the unique haplotypes was very low, especially in Ibiza and the Mediterranean area (Figure 3 / Figure 2; Tomàs article, 2006). Chuetas. A remarkable observation in the Chueta community Nevertheless, the results obtained from the analysis of eight Y- was the high frequency (23%) of preHV-1, a Middle Eastern STRs (DYS19, DYS389I, DYS389II, DYS390, DYS391, lineage that is closely related, though not identical, to many DYS392, DYS393, and DYS385) showed a high level of ge- others found at high frequencies in different Jewish popula- netic homogeneity for eight western Mediterranean populations. The presence of this haplogroup convincingly supported tions. On the other hand, these populations did not show a again the Jewish origin of the Chueta community. The studied compacted group when mtDNA diversity was analyzed, since populations showed a reduced African contribution, and no in- they showed genetic differentiation among them. The analyses dividuals were detected with North African haplogroup U6, in- of haplotypes shared between populations indicated that mtD- dicating a lack of maternal contribution from the Muslim settle- NA haplotypes have drifted to higher frequencies than the Y ment to these populations [18]. chromosome. The haplotypes of Y-STRs did not differ signifi- The study of the genetic variability in the Chueta (Majorcan cantly between the Balearic Islands [23]. The results obtained Jews) and the Balearic (Majorca and Minorca Islands) popula- for the genetic differentiation of the Balearic Archipelago indi- tions was carried out using a multiplex system containing the cate a clear differentiation of Ibiza Island with respect to the nine tetrameric short tandem repeats (STRs) D3S1358, vWA, other Balearic populations, Majorca and Minorca, in autosomal FGA, D8S1179, D21S11, D18S51, D5S818, D13S317 and and mtDNA markers. However, Ibiza does not show differenc- D7S820, again demonstrated that the Chueta population has es when Y-chromosome STRs are considered. Therefore, in remained isolated because intermarriage with non-Jews did not this case, the Y-chromosome markers are less informative for take place until the middle of the twenty century, which has re- tracing the history of the different Balearic populations [23]. We sulted in it being a small inbred community [24]. propose a maternal Carthaginian/Phoenician founder effect for The Chueta community has a particular history, which is dif- the Ibizan population, in accordance with the history of the ferent from other European Jews and which probably molded area, although this is difficult to confirm. Obviously, the genetic their genetic structure. The social discrimination to which they drift generated by this founder effect, as well as by the fact that were subjected and their adherence to tradition restricted the Ibiza is a small island, has affected the genetic differentiation of influx of genes from the host population, but mixed marriages Ibiza Island. This drift has affected mtDNA more than the Y with local population could have taken place more easily than chromosome. In diverse historic periods, the extremely poor mixed marriages between other Jewish European populations economic situation promoted the exodus of men and created a and their host people where there were strict barriers of religion. very stable conserved matrilocal regime. One clear example, Chuetas showed an admixture rate (50%) larger than rates indicative of the few changes in the life of Ibizan women, is that found in other Sephardic Jewish populations but smaller than the feminine wardrobe was not modified on the island until the for instance the rate in Yemite Jews (71%) were conversions to end of the 1970s, whereas the masculine wardrobe changed, Judaism from local people are known [17]. as on the other islands, according to the epoch. The Y-STRs

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in the Mediterranean area, and moderate frequencies in North IBIZA Africa, the Middle East, and parts of Asia (8–10%). In Spanish populations, high H63D frequencies are found (>15%). The distribution of the HFE C282Y and H63D mutations in Chuetas revealed that the H63D mutation was one of the highest frequencies (26.6%) described to date. The frequencies found in MAJORCA the literature in Ashkenazi Jews for the H63D mutation (ap- proximately 9%) and non-Ashkenazi Jews (Oriental, Sephardic and Borth African) arround 12%, differ significantly from that CATALONIA found in Chuetas. An important question is why Chuetas have MINORCA such a high frequency of the H63D mutation. The Chueta pop- ITALY ulation has a particular history—different from other Jews— which has probably moulded their genetic structure. Despite their social isolation, their conversion to Christianity (15th cen- TURKEY 69 tury) allowed mixed marriages with the local population to take 56 place more easily than in other European countries, where TUNISIA there were strict religious barriers between Jews and their host people. In fact, previous studies on classical and DNA genetic markers in Chuetas showed a substantial admixture rate (ap- SICILY proximately 50%). Taking into account this previous genetic in-

ARABS formation for Chuetas, which indicated that they are a hybrid population between Sephardic Jews and Majorcans, we have calculated the H63D frequency expected in Chuetas. The frequencies found in Majorca and Sephardic Jews were 19.5 and 13.3%, respectively. This, combined with an admixture rate of ALGERIA 50%, means Chuetas should have a H63D prevalence of around 16%, which differs significantly from the observed value Figure 3. Tree base on Standard Genetic Distances using the frequen- (26.6%). Therefore, we can discard the fact that the high fre- cies of HLA-A, -B and -DRBI. Only the bootstrap above 50 are represented. quency observed is only due to admixture (Matas, et al., 2006). Genetic drift seems to be the most probable cause for the H63D frequency found in Chuetas. Although there is no histori- showed a high level of diversity and a strong homogeneity cal knowledge about the number of founders of the Chueta among western Mediterranean populations that could be re- population, it is reasonable to assume that the number of lated with numerous male displacements originated by wars Sephardic settlers on the Island of Majorca would have been and commercial relations. The founder effect and the high rate low. In addition, this population suffered different bottlenecks of consanguineous marriages could explain the genetic differ- throughout its history, mainly due to the pursuit of the Inquisi- entiation of the Ibizan population with respect to Majorca and tion. These situations may have led to allelic frequencies differ- Minorca, which is especially evident in the mtDNA. ent from those in ancestral populations. H63D homozygotes genotype must be considered because it confers an increased risk of iron overload and therefore genetic susceptibility to de- Prevalence of hereditary hemochromatosis and veloping HH or to aggravating other diseases [12]. In popula- familial Mediterranean fever in Jewish population: tion of the Balearic Islands diagnosed with HH, the homozygous clinical implications C282Y genotype was the principal one involved in hereditary

Hereditary hemochromatosis (HH) is an autosomal recessive Table 2. Genotype frequencies of C282Y and H63D mutations of HFE trait, frequently found in Caucasian populations, in which the gene in the Balearic Islands excess absorption of iron from the diet leads to severe organ damage. If the disorder is diagnosed before organ damage has Population occurred, reduction of body iron stores to normal will prevent Mallorca Ibiza Minorca Chuetas organ disease and result in a normal life expectancy. Two muta- Genotype N = 192 N = 94 N = 113 N = 71 tions in the HFE gene (C282Y and H63D) were identified as + / + 0.58 0.56 0.68 0.52 being responsible for the disease [7]. Frequencies of HFE + / C282Y 0.05 0.06 0.01 0 C282Y and H63D mutations have been analyzed worldwide. H63D / H63D 0.01 0.05 0.04 0.05 The C282Y mutation is most abundant in North European pop- + / H63D 0.35 0.31 0.27 0.43 ulations and in those of North European descent (allele frequencies of 5–10%). H63D has a much broader distribution, C282Y/H63D 0.01 0.02 0 0 with high frequencies throughout Europe (10–30%), especially N = Number of individuals.

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haemochromatosis (90%), whereas the other HH patiens were [8] Grimaldi, M.C.; Crouau-Roy, B.; Amoros, J.P.; et al. C282Y/H63D compound heterozygous and H63D ho- (2000). West Mediterranean islands (Corsica, Balearic Is- mozygous (Table 1 / Table 2, Guix article) [9]. lands, Sardinia) and the Basque population: contribution Familial Mediterranean fever (FMF) or recurrent polyserositis of HLA class I molecular markers to their evolutionary his- is a hereditary disease characterised by recurrent attacks of fe- tory. Tissue Antigens 58:281-292. ver, transient peritonitis, pleuritis or synovitis, usually lasting [9] Guix, P.; Picornell, A.; Parera, M.; Galmes, A.; Obrador, from 1 to 3 days. The disease occurs predominantly in Jewish A.; Ramon, M.M.; Castro, J.A. (2002). Distribution of HFE populations. The finding of a cluster of FMF patients exclusively C282Y and H63D mutations in the Balearic Islands. Clin among the Chuetas while the disease is unknown among non- Genet 61:43-48. Chuetas inhabitants of Mallorca, raised the question of their [10] Helgason, A.; Hrafnkelsson, J.R.; Gulcher, R.; Ward, R.; possible Jewish origin. The genotype of eight Chuetas FMF Stefansson, K. (2003) A population wide coalescent anal- families showed that three of them had the S haplotype, which ysis of Iceland matrilineal and patrilineal genealogies: evi- is specific for the North-African FMF Jewish patients. One fam- dence for a faster evolutionary rate of mtDNA lineages ily bore the S2 haplotype, which is also common among FMF than Y chromosomes. Am J Hum Genet 72:1370-1388. Jews. Therefore, it is likely that at least part of these two popu- [11] Kayser, M.; Brauer, S.; Weiss, G.; Schiefenhovel, W.; Un- lations (Chuetas and Jews) acquired the FMF gene defect from derhill, P.; Shen, P.; Oefner, P.; Tommaseo-Ponzetta, M.; a common ancestor [6]. Stoneking, M. (2003). Reduced Y-chromosome, but not The human population of the Balearic Islands presents two mitochondrial DNA diversity in human populations from interesting cases when studied from a genetic populational point West New Guinea. Am J Hum Genet 72:281-302. of view: the Chuetas and Ibizans. In both, the founder effect de- [12] Matas, M.; Guix, P.; Castro, J.A.; Parera, M.; Ramon, termined by their origin and the existence of endogamy are the M.M.; Obrador, A.; Picornell, A. (2006). Prevalence of most out-standing aspects. In Ibizan population, a maternal and HFE C282Y and H63D in Jewish populations and clinical paternal differential contribution must be taken into account. implications of H63D homozygosity. Clin Genet 69:155- 162. [13] Merryweather-Clarke, A.T.; Pointon, J.J.; Shearman, J.D.; References Robson, K.J.H. (1997) Global prevalence of putative hemochromatosis mutations. J Med Genet 34; 275-278. 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